melphalan and Amyloid-Neuropathies--Familial

To characterize patients with cardiac amyloidosis using echocardiography, electrocardiogram (ECG) and right heart catheterization (RHC).. Fourteen patients with biopsy verified light chain or transthyretin cardiac amyloidosis were included. All patients had heart failure with markedly elevated NT-proBNP. Echocardiography demonstrated biventricular hypertrophy, left atrial enlargement and normal to slightly reduced left ventricular ejection fraction. Tissue Doppler septal é was low and median E/é was high. Within 6 months RHC was performed in eight of the patients. The restrictive filling pattern demonstrated by echocardiography corresponded well to median pulmonary wedge pressure (21 mmHg). Systolic pulmonary artery pressure (SPAP) was increased, whereas cardiac output and stroke volume were seen to be decreased with both methods. ECG demonstrated: low voltage (36%), abnormal R-progression (65%), ST-T abnormalities (71%) and high incidence of fibrillation (36%). In addition, a case report following the treatment of melphalan and dexamethasone is presented with improvement of hypertrophy, SPAP, left ventricular mass and é.. These findings should lead to a suspicion of cardiac amyloidosis and suggest further investigation.

Topics: Aged; Aged, 80 and over; Amyloid Neuropathies, Familial; Cardiac Catheterization; Cardiomegaly; Dexamethasone; Echocardiography, Doppler; Electrocardiography; Female; Heart Failure; Humans; Male; Melphalan; Middle Aged; Natriuretic Peptide, Brain; Peptide Fragments; Pulmonary Wedge Pressure; Stroke Volume; Ventricular Function, Left